Atypical teratoma/rhabdoid tumor (AT/RT) of the central nervous system is a highly malignant neoplasm in infants and early childhood. Approximately one third of patients develop intracranial dissemination with involvement of cerebral spinal fluid (CSF). The clinical, radiological, and pathological f
Atypical teratoid/rhabdoid tumor of the brain
β Scribed by Parwani, Anil V. ;Stelow, Edward B. ;Pambuccian, Stefan E. ;Burger, Peter C. ;Ali, Syed Z.
- Publisher
- John Wiley and Sons
- Year
- 2005
- Tongue
- English
- Weight
- 467 KB
- Volume
- 105
- Category
- Article
- ISSN
- 0008-543X
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## Abstract ## BACKGROUND: Atypical teratoid/rhabdoid tumors are highly malignant embryonal central nervous system (CNS) tumors that were defined as an entity in 1996. As compared with other malignant CNS tumors, their biological behavior is particularly aggressive, but patients may benefit from a
## Abstract The __SMARCB1__ gene status in 50 patients with atypical teratoid rhabdoid tumor and/or malignant rhabdoid tumor recruited to a German registry was prospectively analyzed with FISH and PCR. Altogether we found 40 __SMARCB1__ mutations in 28 patients. Two patients were positive for SMARC
## Abstract BeckwithβWiedemann syndrome (BWS) is a genetic disorder associated with an increased risk of childhood tumors. Here we describe a patient with BWS who developed a central nervous system atypical teratoid/rhabdoid tumor (AT/RT). To our knowledge, despite the known cancer predisposition,